Intersex Conditions (DSDs) and Pastoral Care. A Guide for Christians. Susannah Cornwall Lincoln Theological Institute The University of Manchester

Transcription

1 Lincoln Theological Institute Intersex Conditions (DSDs) and Pastoral Care Intersex, Identity and Disability: Issues for Public Policy, Healthcare and the Church Briefing Paper 1 A Guide for Christians Susannah Cornwall Lincoln Theological Institute The University of Manchester

2 Intersex Conditions (DSDs): A Guide for Christians This briefing paper has been produced by the Lincoln Theological Institute for the Study of Religion and Society at the University of Manchester, as part of a research project called Intersex, Identity, Disability: Issues for Public Policy, Healthcare and the Church. The paper is designed to outline what intersex conditions (sometimes also called DSDs or disorders of sex development) are and how they have been treated, giving information about the causes and frequency of specific conditions. It is written for those without any specialist medical knowledge. For information about the other papers in the series, see the end of this document. What is intersex? Intersex conditions, sometimes called disorders of sex development, affect roughly 1 in 2,500 people (or about 280 people born in Britain each year). People are often surprised at this relatively high frequency, because in the past intersex was not usually discussed openly, and was often deliberately kept secret. The term intersex covers a range of conditions, all of which entail some kind of atypicality of physical sex. This will vary between individuals according to their specific conditions, but might include: Ambiguous genitalia, which look somewhere between the usual genital appearance of males and females. This might include a structure which looks larger than a typical clitoris but smaller than a typical penis. Note that, contrary to popular conceptions of hermaphrodites, intersex people do not have a full set of both male and female genitalia. A mismatch between genital appearance and some other aspect of physical sex: for example, the vulva of a typical female, but coupled with some male-associated characteristics such as testes rather than ovaries, or XY rather than XX chromosomes. Some other unusual feature of sexual anatomy: for example, one testis and one ovary, or a mixture of ovarian and testicular tissue; or unusual chromosomes (such as XXY, or a combination of XX and XY). 1 P a g e

3 How common are intersex conditions? Estimates put the frequency of ambiguous genitalia at around per cent of births, but the prevalence of hormonal and chromosomal variants is higher, possibly around 2 per cent of births (Preves 2003: 2). This makes intersex conditions much more common than many people realize about as common as a condition like cystic fibrosis. Whilst some intersex conditions, especially those associated with unusual genital appearance, are likely to be diagnosed soon after birth, others may not be diagnosed until later (for example, if a girl does not begin to menstruate as expected at puberty and it is discovered that she has testes rather than ovaries). A fuller outline of the nature, cause and frequency of some intersex conditions is given at the end of this paper. Are intersex conditions hazardous to the health? Ambiguous genitalia do not, in themselves, pose any problems to health. However, certain intersex conditions/dsds are associated with related health risks. For example, some forms of Congenital Adrenal Hyperplasia cause a loss of salts from the body, which can be life-threatening if untreated. However, other forms of the same condition cause unusual genital appearance (such as a large clitoris) but are not in themselves detrimental to physical health. Overall, the majority of intersex conditions/dsds pose little or no risk in themselves to physical health, though it has been suggested that they might pose a risk to mental health if as a result of their unusual bodies individuals do not have a clear sense of gender identity. Some studies suggest that intersex people are more likely than others to experience psychological distress, especially in the areas of anger, mistrust of others, and feelings of inferiority (Kennedy 2003); importantly, however, many commentators believe these problems result from early surgical intervention, and the treatment of intersex people by others, rather than because of the intersex conditions/dsds themselves (Alderson, Balen and Madill 2004; Wilson and Reiner 1998). One woman with Androgen Insensitivity Syndrome says, When I discovered I had AIS the pieces finally fit together. But what fell apart was my relationship with both my family and physicians. It was not learning about chromosomes or 2 P a g e

4 testes that caused enduring trauma, it was discovering that I had been told lies. I avoided all medical care for the next 18 years. I have severe osteoporosis as a result of a lack of medical attention. This is what lies produce. (Groveman 1996: 1829) Why are intersex conditions sometimes called DSDs? Intersex has been used as a term to encompass a range of conditions. However, it was never particularly well-defined, and there has been much disagreement about whether particular conditions should or should not be considered intersex conditions (Dreger and Herndon 2009). Accord Alliance, a North American advocacy group, has called for the use of the term DSD, short for disorder of sex development, rather than intersex. This is because DSD is considered more acceptable to medical professionals and has fewer associations with identity politics. It has increasingly become the term used in medical literature. However, many people with such conditions do not support the use of the term DSD because they do not accept that their condition is a disorder. At present, both terms are used. There are some conditions which are now being called DSDs which would not have fallen within the previous intersex designation because they do not involve either an ambiguity of the genitals or a combination of male-associated and female-associated physical characteristics. These include hypospadias, a condition affecting up to 1 in 250 males, in which the meatus (the hole through which urine and semen are expelled) opens somewhere on the underside of the penis rather than at the tip. Contentious as it is, the term disorder of sex development may be a more useful broad term than intersex in some respects, given that it can encompass a wide range of developmental variations of the chromosomes, reproductive and endocrine systems, not all of which were ever considered intersex conditions. Are intersex people the same as hermaphrodites? The term hermaphrodite is almost never used accurately, and is now considered archaic. Some intersex conditions were formerly called pseudo-hermaphroditism, but this language is now rare. Strictly speaking, the term hermaphroditism refers only to conditions 3 P a g e

5 where an individual has both ovarian and testicular material, and the term ovotestes is generally preferred. People often think of a hermaphrodite as someone who has a full set of male and female genitalia, but this is not actually how intersex conditions manifest. Human foetuses all start off with identical genitals, whether they will develop into males or females. The foetal phallus can develop into either a clitoris or a penis; the original genital opening can remain open as the entrance to a vagina or can close to form the line down the middle of a scrotum. Is intersex the same as transgender? Intersex is not the same as transgender. Transgender people experience a mismatch between their biological sex and their gender identity: for example, a biological female who feels like a man. However, it s believed that most transgender people have no physical ambiguity. By contrast, intersex people have some features in their biological sex which means they can t easily be classified as biologically male or female. Are intersex people homosexual? Intersex conditions are to do with biological sex, not sexual orientation. People with intersex conditions might be heterosexual, homosexual or bisexual, just like anyone else. Why should Christians know about intersex? Intersex hasn t received very much attention from Christian theologians or church leaders. As a result, some intersex people feel invisible in church. Some intersex people have been told by other Christians that intersex is fallen or a result of sin, and not part of God s intention for creation. Intersex Christians may find it difficult to see where they fit in churches which teach that only maleness and femaleness are created in God s image. Most Christians, like most other people in our society, assume that there are only two human sexes, male and female. However, intersex people s bodies don t fit into either box. Some intersex people believe that they are a third sex. Church leaders who assume all people are clearly male or female might not do enough to meet intersex people s specific 4 P a g e

6 pastoral needs. They might also not take into account intersex s implications for Christian teachings about sex, gender and sexuality. Intersex people might have particular pastoral concerns: for instance, some intersex people who had corrective surgery as young children feel they were assigned to the wrong gender, and experience gender identity problems as adults. Some find it difficult to enjoy sexual intercourse because of the surgery performed on their genitals. Others have very negative associations with medical professionals and may feel unable to consult doctors about other medical issues. It can be difficult to provide good pastoral care for intersex people without knowing something about what intersex is. Intersex raises questions about areas of Christian theology, such as teaching on same-sex relationships and gender roles in church leadership. These are explored in more detail in Briefing Paper 4 in this series. How were intersex conditions/dsds treated in the past? Between the 1960s and the 1990s it was common for children born with atypical genitalia to receive corrective surgery soon after birth. This was designed to make their genitals look less unusual, and to promote a clear appearance of male or female sex. At the time, it was believed (basely mainly on work by the American psychologist and sexologist John Money) that it was not possible for a child to grow up with a healthy sense of self if there was any doubt about their gender identity. Money believed that children must be brought up unambiguously as boys or girls, and that their genital appearance should reinforce their gender assignment. Based on a famous case in which David Reimer, one of a set of identical twin boys, was brought up as a girl after his babyhood circumcision went drastically wrong and his penis was destroyed, Money taught that social gender assignment was more significant than biology in developing a clear gender identity. On the basis of this case, many intersex children born between about 1965 and the 1990s with ambiguous genitalia were given corrective genital surgery, either soon after birth or through childhood and adolescence. Surgical techniques meant that it was far easier to remove tissue than to add it. As a result, the majority of children received a feminine gender assignment, since constructing a penis was very difficult. Children whose clitorises were deemed too big, or whose penises were deemed too small, were likely to have this tissue 5 P a g e

7 removed altogether and to be given a vaginal opening and small or non-existent clitoris (Preves 2003: 55). Detailed follow-up studies did not occur, largely because of the belief that they would communicate to children that there had been some question about their physical sex, thereby threatening the stability of their gender assignment. What was wrong with this model of treating intersex/dsd? In the early 1990s, adults in North America who had undergone corrective genital surgery as children began to protest about their treatment and to campaign for delayed or non-surgical intervention for intersex/dsd. In 1996, it was revealed that David Reimer, the boy whose successful reassignment as a girl had been the basis for the widespread acceptance of Money s theories, had in fact never been happy as a girl and had lived as a man since the age of 16 (Colapinto 1997; 2001). Money was discredited, and follow-up studies of other intersex people began to occur. At this time, the older paradigm of early corrective surgery was criticized on several counts by both activists and some medics. These criticisms included: The fact that doctors often did not consult parents of young children, or older patients themselves, about the type of surgery which was being done and why. This secrecy, designed to promote healthy gender identities, was subsequently deemed to compromise agency and to heighten fear and shame among intersex people and their families. The fact that the types of surgery done seemed grounded in patterns which promoted exclusively heterosexual forms of sexuality. For example, it was considered necessary that a boy should have a penis large enough to penetrate a vagina during sexual intercourse, and that if it was not, he was better off having no penis at all. It was considered necessary that a girl should have genitalia capable of being penetrated by a penis, regardless of whether or not these genitalia were themselves capable of feeling sexual sensation (Morris 2004: 26; Kessler 1998: 56). The fact that repeated genital surgery often left genitals so scarred that they were not capable of feeling any sexual sensation at all; the fact that clitoral tissue was 6 P a g e

8 often removed altogether since the clitoris was not considered important to female sexuality. How are intersex conditions/dsds treated now? From the mid-1990s onwards, there have been increased follow-up studies of intersex patients, in order to track whether surgeries done in childhood are perceived by the patients themselves as successful as they grow up. Since the late 1990s, doctors working within the NHS in Britain have been at the forefront of moves to alter the way that intersex conditions/dsds in young babies are treated. Teams based at the University College London Hospitals, and within the Leeds NHS Trust, have pioneered multidisciplinary treatments for intersex/dsd, where patients can consult with a variety of experts in urology, endocrinology, gynaecology, psychology and genetics. Teams of doctors working in the UK and elsewhere have also suggested that much surgical intervention should, if it is to take place at all, be delayed until later childhood. In summary, alternative forms of intervention include: Delayed surgery, following consultation with older children about the implications of conducting or not conducting surgery. Although some forms of intervention become much more difficult after puberty, there is a window of several years between babyhood and pre-puberty when it is possible to discuss options taking into account children s own opinions about their bodies and gender identities. Less invasive surgery, such as hiding rather than removing an unusually large clitoris, so as not to damage the tissue s capacity for sexual pleasure. No surgery: assignment of gender without genital alteration, based on the conviction that it is possible to be brought up as a psychologically-secure boy or girl without having to have typical genital anatomy. In all cases, many medical teams now advocate age-appropriate information for children throughout their treatment, rather than secrecy. Parents and families are now also much more likely to be given details of support and advocacy groups where they can meet and talk with others whose children have similar conditions. Despite the work of these doctors, 7 P a g e

9 however, early and secretive corrective surgery for intersex/dsd still occurs in some hospitals in Britain and many elsewhere. What causes intersex conditions/dsds? There are many different conditions which fall under the intersex umbrella. Some of them are outlined below. These summaries are adapted from Cornwall 2010: Androgen Insensitivity Syndrome (AIS) People with AIS have the testes and XY chromosomes usually associated with males, and the genitals and physical appearance usually associated with females. Most people with AIS are brought up as girls and continue to identify as women throughout their lives. The external genitalia of a typical foetus are not fully differentiated along male or female lines until about twelve weeks gestation (Moore and Persaud 2008: 180). An XY foetus would normally develop male characteristics. The presence of a Y chromosome usually leads to the initial gonad developing into a testis, and the production of a hormone called Müllerian Inhibiting Substance which will block the development of a uterus (Moore and Persaud 2008: 178). However, sometimes an XY foetus cannot respond to androgens produced by the gonads, so the external genitalia develop along female lines. The internal organs, however, develop along male lines. In about two-thirds of cases, this arises from an inherited gene; in the remaining one-third of cases, it arises from a spontaneous mutation. This condition is known as Androgen Insensitivity Syndrome, and can occur in a complete or a partial form. In Complete AIS, the external genitalia appear female at birth, with a clitoris and labia, though the vagina itself may be shallow or absent. Complete AIS affects between 1 in 13,000 births and 1 in 20,000 births. In Partial AIS, some ambiguity of the external genitalia may be present (in other words, the genitals might not look typically female or male). Partial AIS affects about 1 in 130,000 births. In both cases, internally there are testes rather than ovaries, and no uterus. Complete AIS is not usually detected at birth because there is no external ambiguity. However, the undescended testes often result in hernias in infancy which leads to about half of cases being diagnosed. Otherwise, AIS is often not discovered until puberty when the absence of menstruation prompts medical investigation. 8 P a g e

10 At puberty, girls with AIS develop breasts and hips but no pubic or underarm hair. The vast majority of individuals known to have AIS identify as women, and report sexual attraction to men rather than women (Hines 2004: 457). Congenital Adrenal Hyperplasia (CAH) CAH can develop in either XX or XY foetuses. In XX foetuses, it can lead to some genital ambiguity, such as a clitoris which is larger than a typical clitoris, and labia which are fused together. Girls born with CAH who have unusual genitals of this kind have, in the past, often had surgery to alter them. Some critics argue that a large clitoris is not, in itself, a medical problem, and that as long as a child can expel urine and faeces, there is no need for very young girls with CAH to have surgery to open their vaginas (Roen 2008: 56; Creighton 2004: 329). Human bodies produce cortisol, which is essential to survival, regulating energy, blood sugar levels, blood pressure and response to injury. In people with CAH, however, cortisol production is low due to the absence of the gene which converts progesterone to cortisol (Roughgarden 2004: 289). As the body pushes the adrenal gland harder trying to correct the low cortisol level, more and more testosterone is also made. It is this high testosterone level which leads to the masculinization of some girls genitals. CAH can occur in two forms: non salt-wasting and salt-wasting. In the saltwasting form of CAH, if the hormone imbalance is not treated with steroids then lifethreatening loss of salts can occur. Increasing the cortisol level with drugs means the body no longer needs to produce excessive amounts of testosterone. It is the surgery on infants genitals that is the contentious issue, not the treatment of CAH itself. Although salt-wasting CAH in girls and boys requires immediate intervention to replace salts and glucose in the blood, this need not entail surgery to reduce the size of the clitoris in girls. Around 1 in 10,000 people has CAH; an unusual genital appearance related to CAH in girls affects 1 in 20,000 to 1 in 36,000 births overall. CAH is caused by inheriting the affected gene from both parents. Those who inherit it from only one parent will not have CAH themselves, but will become carriers. 9 P a g e

11 5-Alpha Reductase Deficiency (5-ARD) People with 5-ARD appear female at birth but have testes and XY chromosomes. At puberty they may become more typically masculine-looking. Because of the externally female genital appearance prior to puberty, in the past many people with 5-ARD were brought up as girls. However, it is becoming more common for the condition to be discovered early and for parents to be advised to raise the child as a boy, despite the feminine appearance in childhood. This is because the influence of androgens ( male hormones) on the foetal brain is now being emphasized. People with 5-ARD have XY chromosomes but cannot, as infants, convert testosterone into dihydrotestosterone (DHT) due to an absence of the enzyme 5-alphareductase, usually found in the cytoplasm of the cells (Gard 1998: 133). As a result, their external genitalia develop along female lines. However, the uterus and fallopian tubes are absent because the body has still inhibited the growth of the internal female structures as in a typical XY foetus. The testes, epididymis, vas deferens and seminal vesicles are present, though the testes may be hidden inside the body. Although people with 5-ARD cannot convert testosterone to DHT, they are still responsive to testosterone itself (Gard 1998: 137), so secondary male sexual characteristics do develop at puberty. These include growth of the penis, deepening of the voice, increased body hair (including facial hair) (Preves 2003: 29), increased height and muscle mass. If the condition is discovered before puberty and a feminine gender role is preferred, then oestrogen therapy will be recommended at puberty. The testes will be removed to prevent masculinization. However, this means that patients must take hormone replacement therapy to prevent osteoporosis. Genetic Mosaicism People with genetic mosaicism have a mixture of chromosomes in their cells: this could be a combination of, for example, XX and XY, or XY and XO (with only one sex chromosome). Mosaicism happens when an interruption to the cell division early on in the development of an embryo either prevents the expected number of chromosomes from dividing, or creates a mutation in a single gene. The earlier in the process of cell division this occurs, the higher the number of cells which will eventually be affected. 10 P a g e

12 Many people may be genetic mosaics and never realize it because they experience no obvious physical sex ambiguity. However, those with a relatively large minority of different cells may experience some extent of physical ambiguity. Technically, mosaics are any individuals who have patches of cells which differ from the majority of cells in their body, which could have arisen as the result of mutations within a single embryo. The word chimera is sometimes used for people whose tissues originally belonged to two separate embryos that is, when two early embryos fused to form one individual (Dreger 1998: 37). An increase in IVF treatment may lead to a greater instance of this condition: it is common to implant multiple foetuses in the uterus, and two foetuses may then fuse (Strain, Dean, Hamilton and Bonthron 1998). Ovotestes About 1 in 85,000 people has both ovarian and testicular tissue in their body. They may have both a testis and an ovary, rather than the typical two testes or two ovaries. Alternatively, they may have ovotestes, organs containing a mixture of ovarian and testicular tissue. Testicular tissue in ovotestes is thought to be at increased risk of gonadal cancer, so the testicular portion may be removed. The external genitalia may show a huge variation of ambiguity at birth. True hermaphroditism was an older term used for ovotestes, but this is now considered archaic. Sometimes ovotestes may be the result of genetic chimerism; in many cases there is no known medical cause. Where can I find out more about intersex conditions/dsds? The other briefing papers in this series will explore in more detail what intersex means for Christians who are concerned with pastoral care, church policy on sex, gender and sexuality, and theological questions. Further publications from the Intersex, Identity and Disability project at the Lincoln Theological Institute, University of Manchester, will focus on the faith identities of people with intersex conditions. There are also a variety of groups providing information on intersex conditions/dsds and support for intersex people and their families. Contact details for some of these are given below. 11 P a g e

13 Websites providing further information about intersex conditions/dsds: Accord Alliance (US-based): Accord Alliance promotes comprehensive and integrated approaches to care that enhance the health and well-being of people and families affected by intersex conditions/dsds. DSD Families: An information and support resource for families with children, teenagers and young adults who have an intersex condition or DSD. ISNA (Intersex Society of North America) ISNA was one of the first and most influential organizations to campaign on intersex issues. It ceased operating in 2008, but its website, especially the frequently asked questions section, is still a valuable source of information about intersex. UKIA (United Kingdom Intersex Association): An education, advocacy, campaigning and support organisation which works on behalf of intersex people P a g e

14 Works cited Alderson, Julie, Adam Balen and Anna Madill (2004), Fear of Devaluation: Understanding the Experience of Intersexed Women with Androgen Insensitivity Syndrome, British Journal of Health Psychology 9.1, Colapinto, John (1997), The True Story of John/Joan, Rolling Stone, 11 December 1997, 54-97, online at Colapinto, John (2001), As Nature Made Him: The Boy Who Was Raised As A Girl, New York, NY: HarperCollins Creighton, Sarah (2004), Long-term Sequelae of Genital Surgery, in Balen, Adam H., Sarah Creighton, Melanie C. Davies, Jane MacDougall and Richard Stanhope (eds.) (2004), Paediatric and Adolescent Gynaecology: A Multidisciplinary Approach, Cambridge: Cambridge University Press, Dreger, Alice Domurat (1998), Hermaphrodites and the Medical Invention of Sex, Massachusetts and London: Harvard University Press Dreger, Alice Domurat and April M. Herndon (2009), Progress and Politics in the Intersex Rights Movement, GLQ 15.2, Fausto-Sterling, Anne (2000), Sexing the Body: Gender Politics and the Construction of Sexuality, New York, NY: Basic Books Gard, Paul (1998), Human Endocrinology, London: Taylor and Francis Groveman, Sherri A. (1996), Sex, Lies and Androgen Insensitivity Syndrome, letter to the Editor, Canadian Medical Association Journal , Hines, Melissa (2004), Neuroscience and Intersex, The Psychologist 17.8, Kennedy, Kirsty (2003), Investigation of potential mediating factors and presence of psychological distress in people with intersex conditions, unpublished D. Clin. Psychol. Thesis, University of Leeds Kessler, Suzanne J. (1998), Lessons from the Intersexed, New Brunswick, New Jersey and London: Rutgers University Press Moore, Keith L. and T.V.N. Persaud (2008), Before We Are Born: Essentials of Embryology and Birth Defects (Seventh Edition), Philadelphia, PA: Saunders Elsevier Morris, Esther (2004), The Self I Will Never Know, New Internationalist 364, P a g e

15 Roen, Katrina (2008), But We Have To Do Something : Surgical Correction of Atypical Genitalia, Body and Society 14.1, Roughgarden, Joan (2004), Evolution s Rainbow: Diversity, Gender and Sexuality in Nature and People, Berkeley, CA: University of California Press Sadler, T.W ), Langman s Medical Embryology (Eleventh Edition) (International Edition), Baltimore, MD: Lippincott Williams and Wilkins Strain, L., J.C.S. Dean, M.P.R. Hamilton and D.T. Bonthron (1998), A True Hermaphrodite Chimera Resulting From Embryo Amalgamation After In Vitro Fertilization, New England Journal of Medicine 338, Sytsma, Sharon E. (2006b), The Ethics of Using Dexamethasone to Prevent Virilization of Female Fetuses, in Sytsma, Sharon E. (ed.) (2006a), Ethics and Intersex, Dordrecht: Springer, Wilson, Bruce E. and William G. Reiner (1998), Management of Intersex: A Shifting Paradigm, Journal of Clinical Ethics 9.4, P a g e

16 Intersex, Identity and Disability: Issues for Public Policy, Healthcare and the Church Briefing Papers This paper forms part of a series of resources on intersex conditions and Christian theology produced by the Lincoln Theological Institute at the University of Manchester. Briefing Paper 1 outlines what intersex conditions/dsds are and how they have been treated medically, giving information about the causes and frequency of specific conditions. Briefing Paper 2 is designed particularly for people concerned with the pastoral and spiritual care of people with intersex conditions/dsds and the families of intersex children for example, Christian ministers, and those who work in healthcare chaplaincy. Briefing Paper 3 focuses on the implications of the existence of intersex conditions/dsds for the Christian churches policy and teaching on sex, gender and sexuality. It is particularly designed for those involved in reviews of policy on human sexuality and gender, and for social responsibility officers and those involved in equalities and diversity work. Briefing Paper 4 gives an overview of the specifically theological implications of the existence and treatment of intersex conditions/dsds from a Christian perspective. It is particularly designed for clergy and church leaders, those involved in theological education on sex, gender and sexuality, and anyone else interested in theological anthropology. 15 P a g e

17 Version 1 September 2012 Please check for updates on project website prior to distribution Lincoln Theological Institute Department of Religions and Theology School of Arts, Histories and Cultures The University of Manchester Samuel Alexander Building Oxford Road Manchester M13 9PL Intersex Conditions (DSDs): A Guide for Christians 2012 Susannah Cornwall For more information about the Intersex, Identity and Disability project at the Lincoln Theological Institute, University of Manchester, please visit 16 P a g e